Epilepsy syndromes of childhood with sleep activation: Insights from functional imaging

Abstract

In epilepsy syndromes of childhood with sleep activation, defined as the spectrum of epileptic conditions going from classical benign childhood epilepsy with centrotemporal spikes (BECTS) to epileptic encephalopathy (EE) with continuous spike and waves during slow-wave sleep (CSWS) including Landau-Kleffner syndrome (LKS), a lot of functional imaging studies have been performed so far, leading to results that are not always consistent, related to the technique of neuroimaging performed and to the variability of the clinical phenotype. Most consistent findings are, depending of the method used, activations or increased regional glucose metabolism in the epileptogenic regions, and deactivations, hypometabolism or decreased functional connectivity in cortical regions that belong to the default mode network. Functional changes are either transitory, temporally related to the occurrence of interictal epileptiform discharges (IED), or permanent, persisting across IED-free periods. Some studies have shown that the more severe phenotype, i.e. EE with CSWS, displays the more profound functional disturbances. Taken together, functional imaging studies support the concept that IED impact cognition in epilepsy syndromes of childhood with sleep activation. However, the precise chronology between the occurrence of IED and the functional disturbances, the neuropsychological correlates of the functional disturbances, and the effects of the anti-epileptic treatments on IED, functional disturbances and cognition need to be further studied.

Keywords: Centrotemporal spikes; Childhood; Continuous spike waves during sleep; Epilepsy; Functional cerebral imaging.